Objective — the clinical and morphological analysis of aortic lesions in Marfan syndrome (MS).

Materials and methods. 29 cases of MS with aortic aneurysms were observed during the twenty years period (1981-2000). Methods of investigation: echocardiography, aortography, coronaroangiography, pathohistologic examination of operative biopsies (26 patients) and autopsies (6 patients) (H&E, van Gison picrofuchsine, alcian blue, tholuidin blue).

Results: all patients with disturbances of bones and joints system in MS were tall and had arachnodactyly, 15 (51.7 %) of them had deformed thorax. Changes of cardiovascular system were characterized by ascending aorta aneurysms in all patients, including dissecting (93.1 %), of Valsalva sinus (10.3 %), bicuspid aortic valve (68.9 %) and mitral valve prolapse (37.9 %). Marfan syndrome is characterized by aortic dissection with local lesions. Disturbances organ of vision were estimated in 58.6 % of patients; progressive myopіа in 24.2 %, lens subluxation — in 34.4 %. Pathohistologic examination shows injury of medial elastic membranes of aortic wall in all cases. it looked like areas of fragmentation of elastic fibers, that led to generalized lysis (37.9 %). Muscular fibers necrosis was revealed in 51.7 % of patients, total medionecrosis — in 34.4 % cases. and generalized elastolysis, smooth cell collapse of collagen. Changes of aortic valves were expressed in myxomatous degeneration: significant dilation and loosening of spongiform stratum of leaf; fragmentation zones of connective-tissue fibers in superficial dense stratums, signs of sluggish fibrogenesis outside the free edge of leaves. There were garnetting of the fibrous ring of aortic valve, thickening and fragmentation of collagen fibers, that predetermined its dilation.

Conclusions. Marfan syndrome is clinically manifested in young men (20—25 years). Aortic aneurysms dissections with local type of lesion — in aorta ascendance (93.1 %) are the main features of MS. Aortic dissection in MS are based on the injury of elastic membranes in aortic wall media with parts of medionecrosis and generalized elastolysis formation, dystrophic changes and muscular cells necrosis, collagen fibers collapse.

Keywords: Marfan syndrome, medionecrosis of an aorta, elastolysis, aortic aneurysms dissection.

The introduction. The quantity of aortic aneurysm dissection (AAD) grows continuously for last years. A significant part of AAD belongs to medionecrosis of aorta (MNA) — nonatherosclerotic lesion of aorta with cystic wall medionecrosis.

Objective — to define features of macroscopic changes at AAD due to MNA on the basis of complex pathomorphological examination.

Materials and methods. For the period of 1999—2003 66 autopsies of patients with AAD (36 (54.4 %) of them had MNA) were investigated. 28 aortic samples with MNA were studied, complete description of macroscopical changes of an aorta was made.

Results and discussion. Three main macroscopic components are typical for AAD due to MNA: the first one — proximal rupture of intima — an entrance to dissecting hematoma; the second — a cavity of dissection aneurysms essentially — the second pseudo-channel of aorta and the third component — distal rupture of intima, which had connected the cavities of dissection with distal part of aorta that provided circulation in both trunks. Proximal rupture was located in above-valve part of aorta, less often in medium part of an arch; distal — in above-biphurcational position. Dissection was spreaded to backlateral wall more often. Three main variants of dissection were defined: the most frequent one was local, less often — total and subtotal aortic dissection.

Conclusions. Three components of AAD were determined: proximal rupture of intima, cavity of dissection and distal rupture. Three main variants of dissection and two subvariants were allocated. The main diagnostic feature of MNA is double-barreled aorta. Dissection and rupture in MNA, in contrast to other aneurysms, originate without previous dilation of vessel.

Keywords: dissecting aortic aneurism, medionecrosis of the aorta.

In spite of the complete correction of the disease in children with congenital heart diseases (CHD), complicated by pulmonary hypertension (PH), pulmonary artery (PA) pressure (PAP) may be increased during the first day after operation. PAP elevation in some patients is in the form of crisis, which is a complication of the early postoperative period.

Objective: to study the incidence of pulmonary hypertensive crises after surgical correction of CHD, accompanied by PH, to establish the risk factors of PAP elevation and relations between severity of the obstructive disease of pulmonary vessels (ODPV) and the incidence of postoperative complications.

Materials and methods. 42 patients with different CHD, complicated by 100 % PH after correction were studied. The patients age was from 3 to 48 months, mean — 8.2 ± 5.3 months. Patients were divided into three clinical and hemodynamic groups. The first (I) one consisted of 14 (33.3 %) patients with mild clinical and hemodynamic signs of ODPV. The second group (ІІ) consisted of 23 (54.9 %) patients with moderate signs of ODPV and the third (ІІІ) — of 5 (11.8 %) with severe signs. All patients after operation were constantly monitored for PAP during the first 5—7 days. Standard protocol was kept up in all cases.

Results. 37 (88.1 %) patients had episodes of PAP elevation in the early postoperative period. There were 48 severe pulmonary hypertensive crises (SPHC), and 107 — pulmonary hypertensive crises (MPHC). SPHC were registered in 19 (45.2 %) patients, including 1 crisis in 4 patients, 2 — in 7, 3 — in 5, 4 — in 2 and 7 — in 1 of them. MPHC were registered in 13 (31 %) patients. They were easily treated. In 5 (11.9 %) cases PAP remained elevated after operation. 2 (10.0 %) of 19 patients with SPHC had them during the first day after operation, 8 (42.1 %) — the second one, 9 (47.9 %) — the third. SPHC were significantly more frequent in patients of group II in comparison with I and III (р< 0.01). There were no significant differences in SPHC incidence between groups of patients at the age of 3 to 6 months and 7 to 12 months (p> 0.05). At the same time incidence of SPHC in the groups of patients at the age of 3 to 6 months and 7 to 12 months was significantly higher in comparison with patients of >1 y.o. (p<0.05). Level of SPHC was the highest in patients with common truncus arteriosus and total pulmonary veins drainage. Thus, 5 patients with common truncus arteriosus had SPHC in 4 cases, and in 4 of 7 patients with total abnormal pulmonary veins drainage. SPHC were rare in patients with total atrioventricular communication (33.0 %) and VSD (41.2 %). 8 (42.1 %) patients had died during SPHC.

Conclusions. The incidence of PHC in patients with moderate signs of ODPV is significantly higher in comparison with those, who had the mild signes. PHC were not registered in patients with severe ODPV.

Keywords: congenital heart disease, pulmonary hypertension.

The restoration of flow in the infarct related coronary artery not always provides reperfusion of the myocardium, because of distal embolization by thrombotic and atheromatous debris and «no-reflow» phenomenon.

Objective. To assess the influence of distal embolization and «no-reflow» phenomenon during primary coronary interventions on short-term outcome in patients with acute myocardial infarction with ST elevation.

Materials and methods. We assessed the angiograms of 92 patients with acute myocardial infarction with ST elevation, who underwent primary coronary interventions. The angiographically evident distal embolization was presented in 27 patients (29.3 %); the «no-reflow» phenomenon was presented in 2 patients (2.2 %), 31.5 % in total (group I). There were 63 patients without distal embolization (68.5 %, group II). There were no differences between the two groups in age, gender, incidence of diabetes mellitus, hypertension, previous myocardial infarction, ischaemic time, presence of multivessel disease, frequency of myocardial infarction with anterior location, pharmacological therapy, except the use of integrilin (41.4 % vs. 9.5 %, p<0.05). Killip class III-V before primary coronary interventions had 24 (38.1 %) patients without distal embolization and 6 (20.7 %) patients with distal embolization (p>0.05). Stent implantation after initial balloon angioplasty was performed in 7 (24.1 %) patients of group I and 21 (33.3 %) patients of the group II.

Results. TIMI grade 0 in infarct related coronary artery before primary coronary interventions was observed in 26 (89.7 %) vs. 44 (69.8 %) patients (p<0.05); angiographic success after primary coronary interventions (TIMI grade 3, residual stenosis < 50 %) — 20 (69.0 %) vs. 54 (85.7 %) patients respectively (p<0.05); left ventricular aneurysm — 10 (34.5 %) vs. 6 (9.5 %) patients respectively (p<0.05); recurrent ischaemia — 2 (6.9 %) vs. 2 (3.1 %) patients respectively (p>0.05). Left ventricular ejection fraction at discharge was 49.2 ± 1.3 % vs. 44.6 ± 2.5 % respectively (p>0.05). In-hospital mortality was the same in both groups (3.5 % vs. 3.1 %).

Conclusions. The angiographically visualized distal embolization and «no-reflow» phenomenon are presented in 31.5 % of patients with acute myocardial infarction with ST elevation after primary coronary interventions and are associated with worth angiographic results of primary coronary interventions and TIMI grade 0 in infarct related coronary artery before primary coronary interventions. Distal embolization and «no-reflow» phenomenon are associated with higher frequency of the development of left ventricular aneurysm. But they do not significantly impact on the frequency of acute left ventricular failure, recurrent ischaemia and in-hospital mortality.

Keywords: acute myocardial infarction, primary coronary interventions, distal embolization, «no-reflow» phenomenon.

Objective. To determine the influence of lipid tolerance disturbances on the main pathogenic factors of atherosclerosis in patients with ischemic heart disease (IHD) by the test with acute lipid loading results.

Materials and methods. The investigation was performed on 5 healthy volunteers and 25 patients with IHD after uncomplicated myocardial infarction. Total cholesterol (TC) and tryglyceride (TG), cholesterol of high (CHDL), low (CLDL) and very low (CVLDL) density lipoproteins blood contents, modified forms of LDL and VLDL by the data of free radical oxidation (malonic dialdehyde — MDA) and systemic inflammation (C-reactive protein — CRP), glucose and glicosylated hemoglobin (HbA1c) blood contents were determined before and after 4 hours of acute lipid loading — 0.6 g/kg butter intake.

Results. The acute alimentary lipid loading even in healthy volunteers is accompanied by the increase in blood TG contents by 16.0 % (p<0.05) in absence of changes in the level of TC and CLDL. At the same time MDA blood content by 11.0 % (p<0.05) and CRP level — by 73.0 % (p<0.01), level of modified LDL (by 16.0 %, p<0.05) and VLDL (17.0 %, p<0.05) were increased. In spite of the fact, that the blood glucose level was not changed, level of HbA1c was increased by 18.0 % (p<0.05). In patients with IHD the reaction to the lipid loading was much more pronounced in spite of significantly increased initial level of all indices (p<0.05), that was an indication of the lowering of lipid tolerance. Absolute levels of TG and VLDL blood contents increase were 4 times higher in patient's plasma after the loading in comparison with control (p<0.05) in combination with more pronounced increasing of MDA (in 5 times, p<0.05) and CRP (by 76.0 %, p<0.05). Blood level of HbA1c was 4 times (p<0.05), modified LDL and VLDL — 8 and 24 times higher (p<0.001), than in control.

Conclusions. 1. Acute lipid loading induces the development of the complex reaction including lipid, LP and glucose metabolism disturbances — activation of TC and VLDL endogenous synthesis, atherogenic modification of LP, glycoxydation of blood proteins, which is associated with systemic inflammation and oxidative stress. These changes are more full-blown in comparison with healthy persons, which is connected with lipid tolerance disturbances. 2. Combination of these factors and not the separate action of hypercholesterolemia, hypertryglyceridemia or isolated disturbances of lipoprotein metabolism are the reason of aherosclerosis and ischemic heart disease progress.

Keywords: lipids, lipoproteins, glucose, inflammation, oxidative stress, insulin resistance, atherogenesis.

Objective. To establish the role of connective tissue metabolism impairment of large arteries structural and functional changes in patients with essential hypertension.

Materials and methods. 89 patients with stages I—II of Grades 2—3 hypertension (57.4±2.3 y.o.) and 33 practically healthy persons were examined. Morphological parameters of aorta were investigated by MRI with evaluation of external (De), internal (Di) diameters, wall thickness (Hao) of aorta ascendens and the mass of aortic segment (Mao) determining. The functional state of aorta was studied by dopplerography and determining of aortic pulse wave velocity (PWV) and stiffness coefficient of aorta (Cs). Connective tissue metabolism was assessed using blood serum content of free (OPf) and peptide-bounded (OPpb) oxyproline, C-terminal propeptids of procollagen type I (CICP), circulating antibodies to elastin (CAe), and urine content of total (T-AG) and sulfated glycosaminoglycans (S-AG).

Results. We established, that De (2.81±0.05 sm vs 2.53±0.06 sm in control group), Di (3.26±0.06 vs 2.83±0.07 sm in control group), Hao (4.17±0.08 vs 3.02±0.06 mm in control group), Mao (225.4±14.4 vs 132.9±12.1 g in control group), PWV (10.23±0.79 vs 6.72±0.83 m/c in control group) and Cs (11.21±0.81 vs 7.95±0.76 in control group) (all p<0.05) were significantly increased in hypertensive patients and characterized the dilatation of aorta, thickening and stiffening of its wall. Statistically significant elevation of OPpb (11.9±0.65 vs 10.8±0.84 micromol/l in control group), CICP (156.3±11.0 vs 124.3±9.9 ng/ml, correspondingly), T-AG (5.87±0.61 vs 4.29±0.43 mg/g of creatinine, correspondingly), S-AG (2.74±0.41 vs 1.69±0.23 mg/g of creatinine) and decreasing of CAe (16.9±2.46% vs 26.9±2.51% in control group) (all p<0.05) in hypertensive patients due to activation of collagen and proteoglycans synthesis and elastin degradation in essential hypertension.

Conclusions. Significant disturbances of connective tissue metabolism are typical for patients with essential hypertension. They are characterized by activation of collagen and proteoglycans synthesis and elastin degradation, determining structural and functional changes of large arteries in patients with essential hypertension.

Keywords: essential hypertension, arterial vessels, hypertrophy, viscoelastic properties, connective tissue, metabolism.

Objective. To study the impact of amlodipine on the endothelium function, microcirculatory system and oxygen supply of tissues in older patients with essential arterial hypertension (H).

Materials and methods. 40 healthy persons (23 women and 17 men) and 23 hypertensive patients (15 women and 8 men) aged 60 years and over (mean age 73.5±5.5 years) with H of stage II were included in this study. Their basal blood pressure (BP) levels were: 169.7 ± 10.1 mm Hg (systolic) and 100.5 ± 8.5 mm Hg (diastolic). The average age patients' was 73.5 ± 5.5 and disease duration 5.5 ± 0.5 years. Patients were asked to discontinue their hypotensive treatment for at least one week (washout period) before inclusion into the study. After a 7-day washout period the patients began treatment with amlodipine in the dose of 5 mg with the dose increase to 10 mg (20 patients — 87%) one oral tablet daily. For estimation of the treatment efficacy we also studied the 24-hour blood pressure, condition of microcirculation system by the results of capillaroscopy and conjunctival bulbar biomicroscopy; endothelial function, and oxygen supply of the tissues by polarography.

Results. During the treatment systolic BP (SBP) had decreased from 169.7±10.1 mm Hg to 122.2±12.7 mm Hg, diastolic BP (DBP) — from 100.5±8.5 mm Hg to 75.4±10.2 mm Hg (р<0.05). The heart rate (HR) level had increased from 69.0±0.71 to 73.0±0.62 beats/min by 3 months, by 75.0±0.57 beats/min by 6 months, p<0.05 in comparison with baseline. The incidence of capillar blood flow acceleration had increased (from 33.3±3.3% to 65.1±6.5% after 3 and 70.5±5.5% after 6 months). The frequency of stasis had decreased to 20.2±2.1% after 3 and to 15.1±1.2% after 6 months (in comparison with 55.4±4.5% before the treatment initiation). The frequency of homogeneous had increased (baseline — 15.3±1.2%, after 3 — 49.2±4.2%, after 6 months — 60.8±5.7%) and of homogeneous-granular had decreased (baseline — 80.4±6.1%, after 3 — 50.5±4.4%, after 6 months — 29.3±2.4%) (р<0.05). There was diminish of total conjunctival index predominantly because of intravascular component. The level of рO₂ lowering was significantly higher in treated patients during the first (baseline- 3.5±0.2, after 3 — 5.6±1.6, after 6 months — 5.9±1.4) (р<0.05) and the first two minutes of cross-clamping (baseline-7±2.8, after 3 — 10.9±1.6, after 6 months — 12.2±1.8) (р<0.05), indices of latent period of рO₂ increase and time of the level of 75% рO₂ increase accomplishment, time of рO₂ level restoration were diminished after cross-clamping discontinuance. After the intradermal introduction of adrenalin in patients with H in comparison with healthy persons there was more significant spastic reaction (р<0.05): blood flow had decreased by 57.1±2.1% versus 30.8±1.7%, respectively. But after 3 month's treatment maximal reaction of blood flow deceleration was 45.4±2.2%, the reaction took place at 32.4±3.2 min. and all indices were restored by 65.4±5.2 min (all р<0.05 in comparison with the baseline). Such tendency was kept for the next 6 months.

Conclusions. Hypotensive effect of amlodipine (mean dose — 10 mg/day) is accompanied by improvement of endothelium function by the results of evaluation of dermal maximal volume velocity in reactive hyperemia test and intradermal introduction of adrenalin test, and oxygen tissue supply by polarography data in the test with upper extremity vessels cross-clamping, by skin vessels sensibility to adrenaline decrease in aged hypertensive patients. These changes are preserved for 6 months period of treatment. Amlodipine therapy led to acceleration and increase of nail-bed capillary blood flow homogeneity, arteriols dilation and positive dynamics of intravascular conjunctival index because of absence of significant changes in quantity of functioning capillaries by the data of bulbar conjunctival biomicroscopy.

Keywords: microcirculation, endothelium function, polarography, essential hypertension, amlodipine, elderly age.

The modern theories of the pathogenesis of atherosclerosis are analyzed in the problem article, based on literature data and author's investigations. The inflammatory process concerning atherosclerosis is a result of the universal endothelium's response to different disturbing factors (known as common as risk factors) and, possibly, some infectious agents. This view of atherogenesis connected two popular hypotheses — «response to disturbing factors» and inflammatory ones. Infectious agents are not the cause of atherosclerosis, they probably act together with other traditional risk factors. Perhaps, only some infectious agents, due to their protein-lipid content, increased affinity to endothelium, ability to change metabolism of lipoproteins, and may play the role as additional risk factors and probable ethiopathogenetic factors of atherogenesis. Inflammation plays an important role in atherogenesis, but in the case of atherogenesis it has a specialty such as characteristic changes of lipoprotein's metabolism. Normal level of total cholesterol and low-density lipoproteins cholesterol doesn't exclude risk of atherosclerosis development, and more carefully examination of lipoprotein fractions is necessary for establishment of real blood atherogeneity.

Keywords: endothelium, risk factors, cholesterol, lipoproteins.

Significance of chronic protein-energy cachexia is frequently not taking into account in patients with sustained cardiac pathology. At the same time, syndrome of chronic trophic insufficiency is registered in approximately 50 % of patients with chronic heart failure (CHF), and cardiac cachexia (СC) — in 16 %. СC with high probability predetermines the unfavorable prognosis in patients with CHF, and is the reason of complications incidence and mortality increase after cardiac surgery in adults and in children. There is a simultaneous loss of muscular (including myocardial), adipose and osseous tissues because of multipleGfactor neuroendocrinal, metabolic and immune disturbances in the basis of CC. Modern opinions about CC definition, its diagnosis, pathogenesis, methods of prophylaxis and treatment are represented in the article. It was demonstrated, that today the most effective methods of CC treatment are graduated exercise and additional nutrition. The last one is expedient to use in therapeutic and surgical patients. However, methodology of additional nutrition in CC is not elaborated enough. Anticytokine and, probably, gene therapy have significant potential.

Keywords: chronic heart failure, cardiac cachexia, diagnosis, pathogenesis, treatment, cytokines, additional nutrition.

The review of the modern methods of restenoses prevention after endovascular interventions in patients with peripheral arterial disease is presented. Experimental and clinical data about pathophysiological mechanisms of restenosis — elastic recoil, constrictive arterial remodeling and intimal hyperplasia — are summarized. The results of clinical trials of efficiency of stenting, brahytherapy and pharmacological restenoses prophylaxis after endovascular treatment of occlusive lesions of peripheral arteries are systematized. Data about such perspective methods, as photodynamic therapy and cryoplasty, are resulted.

Keywords: peripheral arteries, occlusive lesion, endovascular treatment, restenosis, prophylaxis.